Final decision: thymectomy

Thankfully it’s been a relaxing summer despite the tension in the background while waiting for the results for the tests with the Myasthenia Clinic in Oxford earlier in July. I think there was a spark of denial in me as I was hoping that the blood test would eventually reveal a misdiagnosis (I sometimes still can't believe and accept what I am dealing with...). The blood test measuring the antibodies to the acetylcholine receptor (anti-AChR) which are present in a high proportion of patients with myasthenia gravis has been repeated. Not only that the test hasn't invalidated the diagnosis, but the levels of anti-bodies apparently rocketed to 28 compared to just above 5 in January shortly after the onset (to note that normal levels are considered below 5). I don’t have an explanation yet about what this increment means. All that I can think about is that my thymus is getting enlarged and is secreting more anti-bodies which risk to affect other muscles in high concentration. As well this may be just an obsession I have that something wrong is going on inside me and is out of my control. Unfortunately this kind of tests are not administered very often and I don’t have any scans scheduled in the near future to confirm or infirm my hypothesis.

Paradoxically I don’t feel that my condition has worsen so much over the summer leaving aside the thoughts about denial. On the contrary, I had some really good days when I managed to cope without prisms glasses until late hours of the night. Warm weather didn't seem to affect me despite the predictions from the doctors and other sufferers I've been in touch with on discussion boards and on-line support groups. There seems to be a pattern of good vision in the morning which gradually deteriorates in the afternoon (when muscles and especially ocular ones tend to weaken). The deterioration continues further in the evening to the point that the double vision sometimes can’t be controlled without prisms glasses. I continue to have doubts about the effect of the medication (Mestinon) which does not seem to help too much in the evening despite going back to 3 x 60mg daily.

The bad news is that the results for the other test (Electromyography - EMG) indicated that my muscles did not react normally to the electric impulses which suggests that my condition is in an early stage of generalization. I was afraid that this is going to happen sooner or later and now there is clear evidence supporting the worst scenario (not sure about 'the worst' but definitely is not a happy one). My current physical shape does not seem to match the findings though. I've been feeling well and religiously committed to a healthy life-style with a very balanced diet and regular exercise (five-six times a week). I haven’t noticed any weakness except the overall feeling of fatigue more pronounced in the afternoon (which is a drop in energy levels experienced by most of the people as far as I know). Now if this is related to a generalization of MG is difficult to say as it does not affect a particular group of muscles e.g. limbs. Random twitches of various intensities continue to be very annoying and for some reason usually gets worse after I take the medication (or shortly after the meal following the medication). It is a common side-effect of Mestinon and I wonder whether all the twitching biased the results of the EMG test in any way. I haven’t had the chance to speak to my neurologist about this since I received the results but I’ll definitely ask this question during my next appointment.

Following this change in my circumstances I am feeling more pressured than ever to take some action and make a final decision with regards to the next treatment avenue. As I am not a big fan of medication of any sorts it didn't take me too long to push my decision towards the surgery. I have recently spent a lot of time reading about different treatment options I have at the moment, mainly thymectomy and steroids. I've come across some interesting pieces of research that explore the role of thymectomy in people with ocular or generalized myasthenia. The results sound quite promising although there might be a risk that my reading was 'selective' in order to justify a decision I've almost made before actually the results for my tests came through. Anyway I’ll list below some of the more or less recent articles I found more relevant with a brief description of findings and a link to the source:

Schumm, F., Wiethölter, H., Fateh-Moghadam, A., Dichgans, J.

Thymectomy in myasthenia with pure ocularsymptoms

• The severity of ocular symptoms was rated using a score developed for this purpose. The score progressively declined after surgery to an average of 70% of its initial amount in 80% of patients. Full remission occurred in three cases. No patient developed generalized myasthenia. Antibody titres against acetylcholine receptors if elevated preoperatively also dropped following surgery, with one exception. Clear criteria for the expected therapeutic success of thymectomy could not be identified. Based on our results, and on the assumed significance of the thymus gland for pathogenesis, thymectomy should be considered in patients with pure ocular symptoms.

Roberts, P.F., Venuta, F., Rendina, E., De Giacomo, T., Coloni, G.F., Follette, D.M., Richman, D.P., Benfield, J.R.

Thymectomy in the treatment of ocularmyasthenia gravis

• Transsternal thymectomy (n = 55) and transcervical thymectomy (n = 6) resulted in cure in 31 (51%) patients, improvement in 12 (20%) patients, no change in 16 (26%) patients, and worsening of symptoms (including 1 postoperative death) in 2 patients. Patient outcomes were statistically independent of the duration of preoperative symptoms (mean 9.5 months), patient age, or the presence or absence of thymoma. In patients with ocular myasthenia, 70% were cured or improved after thymectomy; in the subgroup of patients with ocular myasthenia and thymoma, 67% were cured or improved. Thymectomy is an effective and safe treatment for patients with ocular myasthenia gravis.

Liu Z, Feng H, Yeung SC, Zheng Z, Liu W, Ma J, Zhong FT, Luo H, Cheng C.

Extended transsternal thymectomy for thetreatment of ocular myasthenia gravis

• None of the patients experienced a myasthenic crisis, progression to generalized myasthenia gravis, or mortality. Hyperplasia of the thymus was present in 106 of the 115 patients (92.2%). Among 110 patients on whom follow-up was done postoperatively, 29 (26.4%) were in SCR, 64 (58.2%) showed improvement, 7 (6.4%) remained unchanged, and 10 (9.1%) had a worsening of their conditions. The results of the review indicate that ETT is a safe and effective treatment for OMG, especially in patients with illness of shorter duration.

Geoffrey B. Blossom, MD; Raina M. Ernstoff, MD; Gregory A. Howells, MD; Phillip J. Bendick, PhD; John L. Glover 

Thymectomy for Myasthenia Gravis

• Improvement after thymectomy was noted in all 37 patients. Complete remission was achieved in three patients (8%) and pharmacologic remission in 23 (62%). The remainder improved in stage, medication requirement, or both.

Maggi G, Casadio C, Cavallo A, Cianci R, Molinatti M, Ruffini E.

Thymectomy in myasthenia gravis. Results of 662 cases operated upon in 15 years 

• The results of thymectomy in the treatment of myasthenia gravis (MG) arereviewed in the light of a personal series of 662 MG patients, operatedupon during the last 15 years. In 500 MG patients without thymoma, thefollowing results have been achieved: remission 37.9%, improvement 49.4%,unchanged or worse 7.4%, dead 5.2%. There is no sex prevalence and theremission rate is higher in patients under 40 years of age (P less than0.01), with mild disease (P less than 0.05), with a MG duration of lessthan 1 year (P less than 0.05) and with a follow-up length of between 5 and10 years (P less than 0.01). 

And the list goes on and on and on... 

To conclude with, statistically there is a high chance to make some improvement or even go into complete remission following thymectomy. I’ve been offered the video-assisted surgery option which takes longer to perform compared to the trans-sternal one but the recovery is a lot shorter (only a couple of weeks).

I have read about steroids as well and I admit that this would be a quicker and more efficient solution at least on a short term (there is research that claims that the treatment with steroids prevents the generalization of MG). Many participants in research studies or people I have met online have reported a significant improvement following steroids treatment but at the same time they complained about the numerous side-effects which are more noticeable after months of treatment. This is actually the main reason I declined my doctor’s offer of starting a course of steroids. The most common side-effects mentioned by MG sufferers already on steroids are:

• Osteoporosis (thinning of the bones)
• Increased chance of infections (as steroids may suppress the immune system). 
• Increase in blood pressure
• High blood sugar
• Skin problems
• Weight gain
• Muscle weakness (?!)
• Increased risk of developing cataracts
• Increased risk of duodenal and stomach ulcers.

I am perfectly aware that people react differently to steroids and they do not necessarily experience all the above mentioned adverse effects (which may vary with different types and dosages of steroids). However I don’t feel like taking any risks and ending by being further medicated for health problems caused by the steroids.

So here I am, ready to go under the knife! I’ve just been officially referred to a surgeon and now I’m waiting for an appointment to discuss the surgery in detail and arrange a ‘date’ with my thymus... I’m feeling incredibly relaxed overall although I have to admit that all this MG affair hits me really hard when I’m feeling depressed. So far I’ve managed to get up and fight without complaining too much about all these strange sensations in my body and accompanying worries and negative feelings.

Next stop: Oxford!

Oxford is probably the Olympus of Myasthenia Gravis in the UK. It is the place where highly qualified neurologists shed light on this mysterious disease through their widely published research and vast experience. I was lucky enough to be referred to a ‘god’ neurologist with extremely good reviews and qualifications (of course I Googled him prior to my appointment!).

I didn’t know what to expect from this appointment. Definitely not the presence of a couple of research students who observed the ‘patient’ (myself) as directed by the doctor. They have been introduced to me but I haven’t been asked whether I was comfortable or not with their participation. And I wasn’t at all... It was uncomfortable enough to be in a new hospital on my own, consulted by a doctor I didn’t know etc... Looking back I should have probably asked them whether at least I had a choice. My privacy was somehow invaded and I believe that this was the reason I didn’t take enough advantage of this appointment which is probably the most important so far.

Anyway the doctor and I have had the chance to discuss my symptoms which are ocular only at this stage (double vision more pronounced peripherally), side effects (mainly twitching) and the treatment I’ve been taking so far (Mestinon 60mg). The doctor told me that Mestinon does not work very well for people with pure ocular MG. Apparently there are six muscles that control the movement of the eye. Even if Mestinon works 90% and one of the muscles is not targeted for some reason then the double vision will persist. Therefore steroids are more effective in managing ocular symptoms although the side effects are far more numerous and severe. Take for example Prednisone which is a highly popular drug for MG. 

Having previously done some reading about steroids and their impact on health on a long run, I have decided to give them a miss and consider other options - which are quite limited at this stage. Normally the treatment route would be surgery i.e. thymectomy. This is actually the reason I have landed in this well-equipped centre for MG. They perform VATS (which stands for Video-Assisted Thoracic Surgery) which I was explained that takes longer to perform compared to a conventional 'open chest' surgery but the recovery from VATS is much faster. This is because the VATS requires only three small incisions which are minimally invasive. Basically I would need to stay in hospital 48 hours and go back to normal activities in about a couple of weeks. In terms of risks I’ve been told that I might have the surgery done and no improvement afterwards. The chances are that 1 in 4 people are not completely cured or improve at all. However, there seem to be research that shows that thymectomy preformed before the MG ‘explodes’ to other groups of muscles would decrease considerably the chances of generalization. 

My goal for the next couple of weeks is to read some more about thymectomy in Ocular MG as I really want to make an informed decision about the next step. Part of the information would be provided by the results for the blood test (Acetylcholine receptor antibodies) which was repeated and the EMG (Electromyography). I was administered EMG for the first time this month. EMG basically measures muscles’ activity in response to a nerve’s stimulation in order to detect neuromuscular abnormalities. During the test small needles (electrodes) have been inserted through my arm’s skin into the muscle. It was painful at times but not unbearable. I had a chat with the neurologist and found out that the test is very accurate and shows whether MG is generalized or not. Looking forward to the results now!

To conclude, I am in a position to decide whether 1) I continue with the treatment (Mestinon 60mg) I started six months ago, 2) switch to steroids or 3) undertake thymectomy. In case I would decide for the latter, I’ll be further referred to a surgeon who is going to give me more details about the procedure. Their door is open now and all I have to do is to book in. Apparently the waiting list is not too long (which is surprising thinking how long I usually wait for NHS appointments) so I can have this done in weeks. Considering that there is a chance to stop the MG from generalizing to other groups of muscles I would very much like to do it. However I need more time to get some extra information and prepare myself psychologically for this step.

One more thing I would like to add... The doctor I’ve just seen in Oxford is probably the first one to ask me how my condition affected my life. I was pleasantly surprised to be given the opportunity to talk about this but my enthusiasm was short as the doctor didn’t really seem to listen to what I said. He interrupted me repeatedly in the middle of a sentence and changed the subject quite a few times. What can be worse than that?! Well, probably not to be asked at all I presume. Which happened during my last appointment with a neuro-ophthalmologist a couple of days ago. He happens to be the most qualified doctor in double vision in my area but I personally haven’t been impressed first of all by his skills in relating to a patient. At least I was promised that my prisms will be soon incorporated in lens (more aesthetic and more expensive at the same time)! And also I had my Mestinon prescription renewed... However at this stage I am unsure whether improvements in my vision are due to the dosage which was increased a couple of months ago. And when I say that I’m thinking about days when my vision is normal (only when I’m looking perpendicularly) in intervals of time when presumably the effect of the medication has faded away. I’m not taking the third daily dose anymore in the evening (I have discussed this with the neurologist) and honestly I can’t see any difference at all. I haven’t considered going completely off medication though...

P.S. The weather is surprisingly hot these days in the UK but this does not seem to affect me too much. Happy days! 

Up the hill!

I’ve been planning to write this post for a while but I was somehow reluctant to celebrate the slight improvement of my vision over the past few weeks... It’s been so up and down since it had started that I am afraid to even say that it’s getting better just to avoid the disappointment and all the negative feelings caused by a flare up.

I’ve been coping without the prism glasses mentioned in my previous entry most of the time (even when reading or working on computer) over the past couple of weeks. I feel that my vision is now similar to when my symptoms have started. More specifically, I wake up with a brief episode of double vision which fades away relatively quickly (10-15 minutes) regardless whether I take my medication shortly after I wake up.

Therefore I am not entirely sure how much the Mestinon helps me at this stage as my vision is quite good (unfortunately only when I’m looking straight ahead as on sides continues to stay double). To be honest I decided to skip the last 60mg (I take it only twice a day now) as I feel that I can cope with a reduced dose (and my sexual drive seems now back to normal!). I haven’t thought about stopping taking the medication as there is a chance that all the improvement is down to the increased dosage of Mestinon. I should have probably asked my doctor before reducing the dosage but I haven’t had an appointment yet and they told me to take as much as I need anyway (within certain limits of course).

Actually I did have an appointment which I missed because I hadn’t received any confirmation letters from the hospital. You’d be surprised to hear that I have been discharged for that reason so next week I need to chase this up and register again with the Orthoptics. On the neuro side things are going well. I look forward to a couple of appointments at the beginning of July with the Myasthenia clinic in Oxford. I have quite high expectations from the team of specialists in Oxford and I hope that they are going to clarify some treatment routes next month.

Until then I have a holiday planned in the Eastern Europe over the next couple of weeks. This was initially planned in December last year but as I have been unwell I had to cancel it. Temperatures are quite high in Europe around this time of the year and I am aware that the heat can worsen my condition by weakening the muscles. Luckily I live in the UK where the summer usually does not cause any problems (ah, the proverbial British summer!). However there have been a couple of days last week with temperatures higher than usual which have left me depleted and wiped me out especially during late afternoons. Tiredness caused by some restless nights might also have contributed to that.

However my symptoms seem to affect only my eyes at the moment and after 7 months from the onset I think this is a good sign (I continue to pray and hope that it won’t generalize!). I haven’t noticed any radical change in my muscles and the gym continues to be a very good indicator. I dare say that I managed to increase the weights I normally use for my workout (for both upper and lower body) and I’m feeling in a fairly good shape except occasional dizziness, brief double/blurred vision, twitches, headaches, overall tiredness, mood changes, and feeling spaced out at times. 

So far it’s been a step by step approach with a strong focus on here and now which helps me control better the anxiety around my illness and try not too worry more than I should. I am lucky to work term-time only so I basically have the entire summer off to focus on health and hopefully things will improve even more by September when I go back to work!     

From eye patch to prisms

“Try a new pair of glasses! They might sort your vision out!” a work colleague told me and handed me a pair of funny bright yellow sunglasses. She had just bought a fizzy drink and received this free pair of glasses. It might have been a great deal, but I wasn’t in a jokey mood so I politely refused and marked my territory with a fake smile. I usually appreciate jokes as a good laugh may be always therapeutic in dealing with issues that risk to become overwhelming and overly serious. However that day though nothing seemed to work for me; no pair of glasses, no joke, nothing…

I think I gave up hope too soon as I had been offered another pair of glasses the very same day. This time not by one of my colleagues but an ophthalmologist. And it wasn't a pair of cool sunglasses on offer but prism glasses. I had read about prisms on the Internet before and found out how beneficial they can be in correcting double vision. My doctors have never suggested prism glasses so I had to force their hand to explore this option. I thought that after almost six months of appointments I should be provided with a better way to deal with my double vision than an eye patch…

I’ve tried a series of lenses of different strengths and at various angles during that appointment. Some of them made my vision even worse (I didn't even know that that could be possible!) while some others seemed to improve it. Step by step the we came to an optimal solution. They are called Fresnel prisms and apply directly to the spectacle lens. They are not built-in prisms which I found out that are more effective (and expensive as well). I was told that this is going to be a bit of a trial and error. Fortunately so far they seem to help a lot which I wouldn't have expected considering how fluctuating my vision is depending on distances and angles and light conditions etc. It was such a big relief and the feeling was similar to having my usual vision back (at least the frontal one) including the 3D perspective which was obstructed by the eye patch. 

My doctor told me that the prism glasses were mainly for reading and working on computer which I find particularly difficult at work. However after a while I tried them while I was walking and I was pleasantly surprised that they worked brilliantly. Just the occasional headaches and Instagram-like visual sensations due to the light refraction/aberrations (especially in bright sunlight or at night) but hey, no worries, I can live with that! I was relieved to retire my eye patch and embrace a more discreet way to deal with my double vision. This was a real confidence boost especially after my partner told me how sexy I looked in glasses! 

Travelling with MG

This time I am going to write about a real trip – not a metaphorical one. I’ve recently been to Berlin, Germany and my MG behaved really well. The fact that I started the increased dose of Mestinon shortly before that helped a lot. I have to say that I was quite anxious before this trip after I had to cancel my holidays abroad over the Christmas break (just before being diagnosed). At that time I didn’t know exactly who is my travel companion but now we got to know each other quite well despite the constant unpredictability of my illness. I am not going to bore you with the details of a trip which I enjoyed to the maximum together with my partner. I would like to take advantage of the hundreds of pictures I took there and share some fragments of my vision affected by MG. The picture which I processed in Photoshop shows actually how I see when I look straight ahead. There is always a degree of ‘doubleness’ and blurriness which can vary from one minute to another (literally in the blink of an eye). It is sometimes so bad (especially when I walk) that I can’t cope without an eye patch when that happens. I've noticed that the gap between images usually increases if I look at my left/right so maybe you imagine how messed up it can get. Luckily I need to close one eye when I look through the viewfinder of my camera so that comes quite handy! 

More Mestinon...

My next stop on the MG route was at a consultant neurologist specialized in muscle. This happened almost two months after I started the treatment with Mestinon. Apparently the guy had a ‘special interest’ in MG which gave me hopes to find out some more information about my illness from a more credible source than the Internet. Once again I haven’t been seen by the neurologist himself but his assistant was really cooperative and answered all the questions I’ve carefully prepared prior to my appointment. However I can’t say that I was overly happy with the answers as many of them were quite vague. To me that reinforced my worries that the doctors I’ve consulted so far might have a ‘special interest’ in this medical condition but none of them seemed to be specialized in MG. Maybe this explains why I’ve been passed from one neurologist to another every time I’ve been booked in (not to mention the ophthalmologists in between). I dared question their expertise in treating people with MG and kindly asked if I could be seen by someone with more experience in dealing with this illness.

As the treatment I’ve been prescribed did not seem to work for me so far, during the same appointment they decided to double the dose of Mestinon. That means that I am now taking 3 x 60mg per day compared to the previous dosage (3 x 30mg). I’ve been recommended to start with a full dose only in the morning (and keep half the dose in the afternoon and evening). I have tried this approach without any significant improvement after one week. As there were no serious side effects involved I decided to go for the full dose straight away. The good news is that there seem to be more frequent spells of ‘normal’ vision (at least when I look perpendicularly). This happens especially one hour after I take the medication and lasts for about 3-4 hours when the beneficial effects of the medication tend to fade away. Despite this improvement, my vision continues to fluctuate immensely which means that it can get suddenly blurry and/or double depending on distances and the angles I am looking at. However, the fact that over the past few days I coped without my eye patch most of the time made me feel happier and much more confident. I am aware that it can change anytime but instead getting worried and stressed about it I just try to enjoy as much as I can the time I am able to do things pretty much normally.

The doctor who has just seen me told me that he didn't think that Mestinon is going to work and asked me to consider going on steroids (immunosupressants) or more specifically Prednisone. I anticipated this route and luckily I’ve done some research around it over the Internet and asked other patients with MG about this line of treatment on various discussion boards. I was personally put off by the long list of side-effects especially psychological ones (behavioural changes, depression, hallucinations, memory problems etc.). I am aware that everyone’s reaction to medication is different and it is difficult to predict the side-effects beforehand. An argument with the doctors on this issue would have been certainly out of question if I didn’t have a choice. The choice apparently is the thymectomy I was writing about in one of my previous entries. I gave it a thought in the meantime and a good reading and concluded that I would rather prefer surgery to steroids. I have the feeling that surgery will happen sooner or later anyway. Sooner would be better as I am still in a fairly good shape both mentally and physically (my condition has not generalized yet as the symptoms are limited to my eyes only). In the first stage I was told that my thymus was too small to be operated and therefore thymectomy was not recommended. I insisted anyway and asked for more explanations. I was not particularly happy with some vague descriptions like ‘small thymus’. Is thymus supposed to show on a CT scan at all?! I’ve read about many cases of people whose scans were normal and after thymectomy their thymus was huge and inflamed (and sometimes cancerous). The doctor confirmed that most likely the illness is related to my thymus but still he was reluctant about going further with surgery (without any solid arguments). 

Anyway it looks like I’ve made my point clear enough as shortly after my appointment the doctor gave me an unexpected call. He said that considered what we had discussed and would like to further refer me to a MG specialist in Oxford which happens to be one of the best MG centre in the UK. Apparently they perform video-assisted thoracoscopic surgery (VATS) - thymectomy. This procedure requires only very small incisions compared to the old-fashioned ‘open’ surgery which can leave a huge scar on the chest. I have the feeling that this is a step further and certainly good news for me.

I haven’t received any confirmation yet of when and where and with whom the next appointment is going to be. The only letter I’ve received so far was from the consultant neurologist I’ve just seen. Apparently he has booked me in in exactly one year’s time! I hope this is going to be only a follow-up appointment and I will hear from them sooner than that... 

The magic gland

Some things seem to be real only when we are children and tend to disappear by the time we become adults. Thymus gland may be one of them. It grows until puberty and then begins to involute in size and activity as we age. At least it is supposed to unless something goes wrong along the process. For instance, if thymus gland gives incorrect ‘instructions’ to immune cells this sometimes results in autoimmunity. For MG which is an autoimmune disease this means the production of the acetylcholine receptor antibodies which attack the neuromuscular transmission and cause fatigue and weakness in voluntary muscles.

I have to admit that I haven’t heard of thymus before being diagnosed with MG. It seems to be strongly related to MG although the connection between the two remains unclear. Most of the individuals with MG have abnormally large thymus glands which need to be removed before they progress into a tumour called thymoma (which sometimes can be cancerogenous). The surgical removal of the thymus gland is called thymectomy. This procedure proved to significantly reduce symptoms in individuals with MG (with or without thymomas). Some research claims that 70% of patients undertaking thymectomy reported reduced symptoms or even complete remission after a number of months/years.

Shortly after I have been diagnosed I was referred for a CT for chest (where this gland is located – just below the neck, behind the breastbone and in front of the heart). I have just received the results and I am not sure whether it is good or bad news... I just take them as they come in a detach way most of the time. The scan has NOT shown any particular abnormalities or at least not to the extent that requires surgery: "CT scan of the chest shows a small degree of tissue in the anterior mediastinum. This may simply represent a small remnant of thymus gland. Whether it is relevant to his symptoms of myasthenia is unclear. Certainly with myasthenia limited to the ocular muscles we wouldn't tend to advocate thymectomy...". However I have read about people with MG who had their thymus gland removed even if it wasn't enlarged (or at least wasn't visible on CT/MRI scans). I don’t even want to think about it right now as I need to work out my beach body for my exotic holidays later this year! Leaving the joke aside, I definitely have to ask more questions about this (and many other things) during my next appointment with my neurologist in a couple of weeks…